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Your new baby will have many firsts: bath, feeding, and of course, dirty diaper. But what happens if a day or two goes by and your newborn doesn’t have that first bowel movement? If this happens, physicians might consider your baby for Hirschsprung’s Disease.

Hirschsprung’s Disease (HSD) is a rare disease caused by an abnormality in the lower part of the colon, often leading to a partial bowel obstruction because the colon is unable to relax. When the colon forms between week five and 12 of gestation, certain cells fail to migrate into position, leading to a colon that does not function as it should.

HSD can run in families, but it can also occur in children with no family history of the disease. It occurs in approximately 1 out of every 5,000 births, with a male predominance of nearly 4 to 1.

Most children with HSD show symptoms within the first few days to weeks of life. Infants who do not pass meconium, a black tarry substance, within 24-48 hours of birth should always raise a red flag that something serious is going on. Infants can also have vomiting, a swollen abdomen, infrequent bowel movements, and difficulty feeding. Sometimes patients who are not diagnosed in infancy will show the disease in early childhood with chronic constipation and poor weight gain.

Early diagnosis is important to prevent serious complications, such as rupture of the colon or a severe infection known as enterocolitis. If your child’s doctor suspects HSD, he or she may order a barium enema to look at the anatomy of the colon. Your child may also be sent to a pediatric surgeon, who will perform a suction rectal biopsy for definitive diagnosis.

If HSD is confirmed, your child may need a surgery to remove the affected, nonfunctioning part of the colon. This may involve a procedure called a “pull through” where the abnormal section is cut out and the normal colon is pulled through to the rectum or anus. In severe cases, where there is a large section of colon involved, your child may need a temporary colostomy, which diverts the colon to an opening on the skin, where stool is collected in a bag.

With proper diagnosis and treatment of HSD, many children live normal, healthy lives. Some may continue to suffer from constipation, and all remain at risk for enterocolitis, the severe colon infection, for many years postoperatively.

Reviewed by Dr. Sara Connolly, December 2018

Takeaways

  • An abnormality in the lower part of the colon that could lead to a partial bowel obstruction is Hirschsprung’s Disease.
  • Most children with Hirschsprung’s Disease show symptoms within the first few days of life.
  • IF HSD is confirmed, surgery may be required to fix the affected area.

References

  1. Pediatrics. Hirschsprung’s Disease: A Review.
  2. Pediatrics in Review. In Brief: Hirschsprung Disease.
  3. American Family Physician. Hirschsprung’s Disease: Diagnosis and Management.

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